After Creutzfeldt-Jakob disease and mad cow disease became rampant a few years ago, it had become quieter about these diseases. But there is now a new variant of Creutzfeldt-Jakob disease (vCJD), the human form of BSE, so to speak, which insidiously begins with atypical symptoms.
What is the new variant of Creutzfeldt-Jakob disease?
No reported case of vCJD exists in Germany yet, but there are about 200 cases worldwide, most in the United Kingdom. While the actual Creutzfeldt-Jakob disease mainly affected older people, the age of vCJD is much lower. To curb the spread of vCJD, various consumer laws have been enacted, such as BSE testing of all slaughtered cattle over a certain age limit. The new variant of Creutzfeld-Jakob disease is caused by malformed proteins (prions) that can cause brain damage. The symptoms of vCJD closely resemble those of Creutzfeldt-Jakob disease. This type of disease is one of the “transmissible spongiform brain diseases.” They are also called prion diseases because they are caused by prions. The first case of Creutzfeldt-Jakob became known in 1995. About a year later, links were recognized between this disease and mad cow disease (BSE), which occurs in cattle. In 1997, evidence was obtained that prions were responsible for both BSE and Creutzfeldt-Jakob disease.
Causes
The new variant of Creutzfeldt-Jakob disease is triggered by defective proteins (prions) found in nerve and immune cells. It is not clear why they develop. It may be due to gene mutations. The prions have a different sequence of amino acids than normal proteins. It has not yet been clarified how the prions damage the brain. Since they also do not trigger defense reactions, diagnosis is very difficult.
Symptoms, complaints, and signs
New variant Creutzfeldt-Jakob disease does not have very specific symptoms at the beginning of the disease. There are mood changes and onset of anxiety in affected individuals. These may be periodic at first and then manifest. Depressive moods are common. Hallucinations occur. The first motor sign today is unsteadiness when walking. As the disease progresses, there is a loss of concentration and a steady decline in cognitive performance. Those affected suffer from an increasingly deteriorating memory and thus show the symptoms of dementia. This is also accompanied by all the other symptoms of dementia – such as disorientation and sudden aggressiveness. The ability to read, write and speak can be completely lost. There is sometimes complete apathy. vCJD leads to other motor symptoms. There is uncontrolled trembling. Movements become more uncoordinated and may appear particularly expansive or as if paralyzed. The muscle disorders also cause sudden movements and can further limit speech.
Diagnosis and course
To date, there is no diagnostic procedure that reliably detects new Creutzfeldt-Jakob disease. Symptoms and complaints are also not unequivocally attributable to vCJD and may resemble those of other diseases. Somewhat more certainty can be gained by magnetic resonance imaging (MRI), which would reveal damage to brain tissue in affected individuals. To detect prions, a tonsil biopsy can be performed. It is a particular risk if people have contracted the new Jakob-Creutzfeldt disease but do not yet have any symptoms. If they donate blood, the prions can be passed on. That’s why researchers are working harder to be able to detect the vCJD pathogen through blood tests. The difficult thing is that in the early stages of vCJD, there are no characteristic symptoms that allow a conclusion to be drawn about vCJD. The symptoms are rather unspecific, such as depressive moods, anxiety, concentration disorders, and a decline in mental performance. In addition, movement disorders may occur and uncontrolled tremors. In the further course, paralysis, muscle twitching and uncontrolled movements may occur. Increasingly, the ability to read and speak gets out of control.In the final stage, vCJD sufferers can no longer communicate with their environment at all. They become apathetic, eventually unconscious, and eventually die.
Complications
Complications of new variant Creutzfeldt-Jakob disease (vCJD) arise in direct relation to the symptomatology of the disease. They occur both with and without treatment of the disease, for which there are no fundamentally effective drugs, only symptom-relieving ones. Psychological symptoms such as confusion, anxiety or depression often initially lead to misdiagnosis at the beginning of the disease. Also, after only a few months, patients are often no longer able to cope with normal everyday life on their own. Motor disorders such as gait instability or uncontrolled falling also increase the risk of accidents as a complication in the course of the disease. Movement disorders and epileptic seizures are typical as the disease progresses and can also lead to accidents and injuries to patients as complications. Urinary and fecal incontinence and progressive dementia characterize the more advanced new variant of Creutzfeldt-Jakob disease and ultimately necessitate intensive care and nursing of the sufferers around the clock. In the final stage, patients are then no longer able to make contact with their environment. Often they are in a state of complete spastic paralysis (so-called entrainment rigidity). In this terminal state, respiratory paralysis or pneumonia often occur as complications. These then lead to the death of the patient in most cases.
When should you see a doctor?
If irregularities of well-being arise, special care should already be taken with regard to one’s own sensations. In the case of a diffuse experience of anxiety, changing moods or a peculiarity of behavior, the first signs of a health disorder are already apparent. If there is a depressive appearance, a withdrawal from social life or a breaking off of close contacts, there is cause for concern. This is especially true if there are no incidents that could explain such a decision. Observations and changes should be discussed with a physician so that a follow-up examination can be initiated. If there is a decrease in mental performance or weakness in concentration, consultation with a physician is indicated. If the complaints persist over a longer period of time or increase in intensity, medical help is needed. Clarification of the cause is necessary so that a treatment plan can be established. Aggressive behavior, changes in personality, and loss of memory should be investigated. If there is disorientation or loss of learned skills such as reading, writing or arithmetic, the affected person needs help. If there are disturbances of the muscular system, an unnatural decrease in physical resilience, and trembling limbs, a visit to the doctor is necessary.
Treatment and therapy
The new variant of Creutzfeldt-Jakob disease cannot yet be effectively treated with drugs. However, medical researchers are conducting intensive research to find drugs that can successfully combat vCJD. So far, those suffering from vCJD are treated with various drugs that can delay the progression to some extent. Muscle twitching can be treated with appropriate drugs. However, since there is no drug for vCJD on the market, vCJD is still fatal so far. The average life expectancy after contracting vCJD is about 14 months.
Outlook and prognosis
New variant Creutzfeldt-Jakob disease has an unfavorable prognosis. The health disorder is characterized by a highly progressive course. Despite all efforts, no effective therapy has yet been found to halt the progression of the disease. Regardless of whether treatment is initiated or the affected person forgoes it, serious health irregularities occur that contribute to a diminished quality of life. Cognitive performance is severely degraded, leading to the need for intensive care. Ultimately, after a severe course of the disease, the affected person passes away prematurely. Statistically, the patient is predicted to have a life expectancy of about 14 months after diagnosis.In addition to the cognitive problems, motor irregularities appear within a few months. If the course of the disease is favorable, drug therapy delays the progression of the disease. However, this cannot be achieved in all patients and depends on the stage of the disease. Behavioral changes and a transformation of the personality are to be expected. There are problems with orientation, states of confusion and immense memory loss. The disease is therefore difficult to endure for the patient and especially for his relatives. In most cases, due to the severity of the symptoms, inpatient treatment becomes necessary at an advanced stage of the disease.
Prevention
Just as there is no cure, there is no prevention of vCJD. While there are consumer protection regulations that say beef and products made from it must be carefully sourced, it is not a guarantee. Cooking does not help either, because the prions cannot be destroyed due to their heat resistance. However, since January 2001, there has been an EU decision that all slaughtered cattle intended for human consumption must be tested for BSE.
Follow-up
There is little follow-up for the new variant of Creutzfeldt-Jakob disease, as the disease is not curable and cannot be treated to any significant degree. It always ends with the death of the affected person, and spontaneous cures have not yet occurred. Thus, medical follow-up is not expected to play a role in vCJD in the future. Also, the many symptoms of brain failure are not treatable and thus do not provide a basis for follow-up care. It is much more important to provide adequate care for the diseased person – especially in the last months of his or her life. However, the rare cases of this disease sometimes bring new insights regarding the pathogens as well as the possible ways of transmission. Research into Creutzfeldt-Jakob disease and the prevention of further outbreaks can therefore be seen as aftercare. More important is the psychological aftercare or care of the relatives. Since the new variant of Creutzfeldt-Jakob disease more frequently affects comparatively young people, the shock often runs deep, especially for the parents. Accordingly, relatives should not be left alone after the death of the patient and should be able to call on help if they so wish. This depends very much on the individual case. An understanding of the disease can help here in overcoming the loss.
What you can do yourself
There are no self-help measures that would somehow counteract New Variant Creutzfeldt-Jakob Disease. All possibly advised self-help attempts by questionable substances, homeopathic remedies or alternative therapies have so far led to no positive effect. The disease, once erupted, is considered incurable. The only positive aspect that can still improve the quality of life of those affected is the fact that the course of the disease is well known. If the first symptoms appear, those affected know what is in store for them. It is then up to the patient himself how he wants to use his remaining months. Since patients can still live an almost normal – albeit limited – life for several months despite their symptoms, they are well advised to make full use of this time. Especially in the early stages, which are characterized by anxiety and mood swings, human support is all the more important. The quality of life of those affected is improved by responding to them. Activities should be taken up. As long as they are still able to move, they should be encouraged to do so. The further stages, which are also accompanied by pain, also offer no scope for medical self-help measures. Here, too, only human attention comes into consideration.
